Spina Bifida

With the Centers for Disease Control (CDC) saying that one of every 2,500 babies born in the United States is diagnosed with this condition, spina bifida is, according to the Spina Bifida Association, “the most common permanently disabling birth defect in the United States.” The latest CDC statistics say that 154,000 people in the United States are living with spina bifida, a total much higher than previously thought.

What Is Spina Bifida?

The National Institute of Neurological Disorders and Stroke (NINDS, part of the National Institutes of Health) defines spina bifida as “a neural tube defect — a disorder involving incomplete development of the brain, spinal cord, and/or their protective coverings — caused by the failure of the fetus’ spine to close properly during the first month of pregnancy.”

At birth, “Infants born with spina bifida sometimes have an open lesion on their spine where significant damage to the nerves and spinal cord has occurred,” NINDS says. “Although the spinal opening can be surgically repaired shortly after birth, the nerve damage is permanent, resulting in varying degrees of paralysis of the lower limbs. Even when there is no lesion present, there may be improperly formed or missing vertebrae and accompanying nerve damage.”

There are several different types of spina bifida, generally identified by the level and location of the injury. NINDS defines them as follows:

Myelomeningocele: The spinal cord and its protective covering (the meninges) protrude from an opening in the spine; this is the severest form of spina bifida.

Meningocele: The spinal cord develops normally, but the meninges protrude from a spinal opening.

Occulta: One or more vertebrae are malformed and covered by a layer of skin. This is the mildest form of spina bifida.

In addition, NINDS notes that children with spina bifida often have a condition known as hydrocephalus — excessive cerebrospinal fluid in the ventricles around the brain — and often have learning disabilities. Difficulty in controlling bowel and bladder functions is also common, and children with spina bifida are also prone to latex allergies.

The cause of spina bifida is not currently known, though researchers have found some higher-risk factors, including obesity in the mother prior to pregnancy. The CDC noted, “There has been a 24-percent decline in babies born with spina bifida since the United States began fortifying grains with folic acid.” The CDC therefore now recommends that women of childbearing age consume adequate amounts of folic acid.

“Typically, children with spina bifida will have difficulty with mobility,” she says. “In lower-level lesions, children are expected to ambulate to some degree, but acquisition of this mobility is delayed. Therefore, mobility devices such as manual or power wheelchairs may be prescribed.”

In addition, spina bifida clients can be expected to have altered sensation, and a number of patients will have hydrocephalus. The hydrocephalus, Meyer notes, “often is treated with a Ventriculoperitoneal (VP) Shunt, which drains the excess fluid into the child’s abdominal cavity, where it is absorbed by the body. If this fluid imbalance creates too much pressure on the brain, it could cause damage to the brain, resulting in additional neurologic insult potentially affecting mobility — i.e., tone changes, balance issues, etc.”

Assistive Technology Interventions

Patients with spina bifida can potentially benefit from a range of equipment, Meyer says, including…

“Skin inspection and pressure relief are especially important both at the seating surface and in all areas with altered sensation,” Meyer says.

She also adds that intervention should happen early to give the child with spina bifida every chance to become independently mobile as soon as possible.

“I promote doing this at a very young age — supported by Cole Galloway’s research at the University of Delaware (see sidebar) — to promote independent exploration, which is essential for development,” Meyer says.

At the same time, seating & mobility providers should be on the lookout for certain clinical issues when working with this population.

“Some challenges we need to be aware of when prescribing mobility equipment for these children,” Meyer says, “are reducing the risk of shoulder-overuse injury associated with lifetime wheelchair use and addressing the need for multiple types of mobility equipment, including orthotic management, ambulation aids such as crutches or walkers, and a wheelchair. Positioning challenges are usually mild unless the child has significant spinal deformity, but we must be sure to appropriately set up their equipment to preserve upper-extremity function.”

While children with lower-level spina bifida may eventually learn to walk, Meyer cites studies that measured the often extensive amounts of energy that these children need to expend to ambulate. The studies showed that children with spina bifida use much more energy to walk than their able-bodied peers need to.

In one study mentioned by Meyer (Williams, et al, 1983 as cited by Campbell SK, 1994), researchers found that ambulation by children with spina bifida was 218 percent less energy efficient than the walking done by able-bodied peers. The study also found, Meyer says, “Energy expenditure was significantly lower during wheelchair propulsion than during walking,” and “Wheelchair propulsion was as fast and as energy efficient as normal walking.”

Another study that Meyer cited (Franks, et al, 1991) said that children with myelomeningocele spina bifida spent 42 percent less energy to propel their manual wheelchairs than to walk with crutches at the same speed.

A 1981 study (Evans EP, Tew B.) quoted by the U.S. National Library of Medicine observed 22 children who had spina bifida and studied their walking and wheelchair propulsion.

“Walking energy expenditure was generally higher than during wheelchair ambulation and significantly higher again than that expected for normals matched for weight,” the study noted. “The energy expended during both types of locomotion related to the weight of the subjects and not the site of lesion.”

The take-away from such studies as these, Meyer suggests, is “that these children need to have a means of functional, efficient mobility that doesn’t utilize all of their energy — and allows them to fully participate in life as their peers do.”

The study quoted by the U.S. National Library of Medicine report also noted, “Physical apathy, excessive weight and increased energy expenditure tended to be connected.” In a child’s terms, that could be interpreted as “If I have to spend all my energy to walk across the playground to reach a group of my friends, but I’m exhausted when I get there, how much fun can I have? And will I want to walk all that way next time, if I get so tired that I can’t play with them?”

Addressing Social, Cognitive & Emotional Needs

“Like all patients, children with spina bifida need to be treated holistically, and family involvement and support are a must for good follow-through,” Meyer says.

She’s noticed some social and emotional tendencies among children with spina bifida.

“In general, kids with (myelomeningocele spina bifida) have difficulty with separation from parents — partly due to parents having a difficult time letting these children be independent (so they) may do more for them than necessary,” she says. “Also, another thing I have noticed is the difficulty separating from the equipment — i.e., changing from one wheelchair to a new wheelchair — even if it is nearly identical to the current chair. Their mobility equipment truly becomes an extension of their body, and it is hard to part with something that has helped them be independent for so long.”

While there currently is no cure for spina bifida, the right assistive technology introduced at the right time can go a long way toward helping children with this condition to be as independent as possible.

The Impact of Early Intervention on Mobility

As referenced by Permobil Pediatric & Standing Specialist Amy Meyer, PT, ATP, the University of Delaware has been studying the impact that mobility technology can have on infants and very young children who, due to medical conditions or injuries, would otherwise be delayed in attaining independent mobility.

Among the researchers involved are Cole Galloway, Ph.D.; Amy Lynch, Ph.D.; Ji-Chul Ryu, Ph.D; Sunil Agrawal, Ph.D; and Christina Ragonesi, a graduate student working on her Ph.D. The setting: the University of Delaware’s Early Learning Center, where a pintsized robotic power wheelchair called the UD2 is enabling children to independently move around and explore their world, months or years earlier than they might have otherwise been able to.

The program itself is exploring the possibilities of what could happen if a child with a developmental or physical delay — for instance, Down’s syndrome — is given access to independent mobility via that tiny power chair. If the child could learn how to operate the chair and thus explore on his own, would his cognitive, social and emotional development benefit?

Perhaps the study’s best-known participant is Andrew Peffley, who began learning to operate a power chair at age 7 months. By giving Andrew, who has spina bifida, repeated and regular access to a power chair at that young age, they hoped to offer him independent mobility at a time when an ablebodied infant would begin to attain it by rolling, sitting up, crawling, etc. The general idea is to replicate the mobility developmental process as closely as possible for kids with disabilities.

Galloway has called this practice “mobility immersion,” and has been quoted at the University of Delaware as explaining, “It’s kind of analogous to an undergraduate learning Spanish by being dropped off in Spain.”

For more info on this ongoing research, visit the University of Delaware’s Web site: udel.edu/research/media/babiesrobots.html. To download and read the University of Delaware case report on Andrew’s great adventures, use Google to search for “university of delaware power mobility” and then click on the article called “Power Mobility Training for a 7-Month-Old Infant with Spina Bifida.” Mobility Management also reported on this study in its May and June 2009 issues; go to mobilitymgmt.com to check the issue archives.

Spina Bifida Resources

Spina Bifida AssociationSpinaBifidaAssociation.orgCreated to enhance the lives of people with spina bifida and to encourage advocacy and research, the Spina Bifida Association has 125 nationwide chapters to serve clients in their communities. Its National Resource Center on Spina Bifida is housed in the organization’s Washington, D.C., headquarters and includes white papers on topics such as the effects of folic acid during pregnancy, educational issues that impact children with spina bifida, and depression and anxiety in people with spina bifida. Download these papers and fact sheets online by going to SpinaBifidaAssociation.org, and clicking About Us/About SBA/National Resource Center on Spina Bifida.

First World Congress on Spina Bifida Research & Caremedicalconference.SpinaBifidaAssociation.orgThe March 2009 event for medical professionals brought together international specialists in the fields of neurosurgery, neurology, developmental pediatrics, orthopedics and urology. Abstracts from the event are available for download from the Web site. On the mobility side, abstract topics include “Advances in the Management of Scoliosis in Spina Bifida,” “Performance of Activities of Daily Living in Children Born with Spina Bifida,” and “Hip Dysplasia in Children with Myelomeningocele: Efficacy of Treatment.”

37th Spina Bifida Association National ConferenceSpinaBifidaAssociation.orgTakes place in Cincinnati, June 27-30. The event is attended by spina bifida patients and their families, as well as health-care professionals, and emphasizes advocacy, education and networking.